Impact of pregnancy in patients with systemic lupus erythematosus-associated pulmonary arterial hypertension: case series and literature review

Objective This study aimed to investigate the clinical characteristics and outcomes of pregnancy complicated by SLE-associated pulmonary arterial hypertension (SLE-PAH) in a case series and literature review.Methods This single-centre retrospective study included 10 consecutive pregnancies complicated by SLE-PAH confirmed by right heart catheterisation (RHC) at Peking Union Medical College Hospital between 2009 and 2020. A literature search was conducted and 14 pregnancy cases complicated by SLE-PAH were reviewed.Results At the time of 10 patients’ initial visits, the average age was 30.00±5.72 years and the median disease duration of SLE and PAH was 34.5 (range 1–164) months and 2 (1–51) months. Two patients carried planned pregnancy, seven patients developed PAH during pregnancy and one pregnancy was unplanned. Further, nine patients had low disease activity, with Systemic Lupus Erythematosus Disease Activity Index between 0 and 4, and 30%, 30% and 40% of patients were of WHO functional class II, III and IV, respectively. All patients were evaluated by RHC and echocardiography. N-terminal pro-brain natriuretic peptide (NT-proBNP) levels were elevated in 70% of patients, with a median level of 776 (56–18 023) pg/mL. The median time of completed pregnancies in all patients was 31 (15–38) weeks and six patients delivered live infants. SLE activity and PAH severity improved in 70% of patients within 6 months after delivery. One patient died on the 15th day after induction of labour. In the remaining patients, all achieved a lupus low disease activity state; according to the European Society of Cardiology/European Respiratory Society risk stratification, seven were categorised at a lower risk state compared with their risk stratification during pregnancy, and two remained at intermediate risk. Additionally, 80% of patients exhibited mild impairments with WHO functional class I or II. The median NT-proBNP level was 184 (32–4003) pg/mL within 6 months after delivery. In the reviewed literature, the average age of patients was 30.09±5.37 years. The median time of completed pregnancies was 36 (28–40) weeks. More cases were planned and successful, and the survival rates of mothers and neonates were 85.71% and 92.86%, respectively.Conclusions Successful pregnancy could be possible in women with SLE-PAH if SLE-PAH treatment goals are achieved under proper therapies, careful monitoring and thorough evaluations.